Adams-Oliver syndrome.

A new-born male baby with typical features of Adams-Oliver syndrome (AOS) is described. Adams-Oliver syndrome is the association of aplasia cutis congenita with terminal transverse limb reduction defects with or without cutis marmorata telangiectatica congenita. The patient presented with brachydactyly involving all the digits of his hands and shortening of both big toes along with aplasia cutis on the scalp. There was no systemic involvement. The patient was placed on regular follow-up.

Vitiligo appearing in striae distensae as a Koebner phenomenon.

Koebner phenomenon, the appearance of preexisting skin lesions following trauma to previously uninvolved skin, has been seen frequently with vitiligo. The type of trauma leading to Koebner phenomenon can vary from scratching, surgical scars, radiotherapy, burns, irritation from drug use and laser therapy. Striae distensae are a form of injury to the skin and in this particular case resulted from rapid increase in body size at puberty and possibly the use of systemic steroids for the treatment of vitiligo. The appearance of vitiligo in striae distensae as a form of Koebner phenomenon has been reported rarely. We describe a 14-year-old boy with vitiligo for the past 2 years, which Koebnerized in the striae distensae.

Clinical efficacy of intramuscular meglumine antimoniate alone and in combination with intralesional meglumine antimoniate in the treatment of old world cutaneous leishmaniasis.

Treatment of cutaneous leishmaniasis is often difficult. Even though most cutaneous lesions will heal spontaneously, their duration cannot be predicted in an individual case. In general, only large, multiple or diffuse lesions of the face, head and neck need to be considered for therapeutic intervention. Pentavalent antimonials (sodium stibogluconate and meglumine antimoniate) administered intralesionally or parenterally (IM or IV) are the mainstays of systemic therapy despite the toxicity associated with their use. The objective of this study was to compare the clinical efficacy of intramuscular pentavalent antimonial compound meglumine antimoniate alone and in combination with intralesional therapy in the treatment of Old World cutaneous leishmaniasis. Study was conducted as a case controlled interventional prospective study. On the basis of demonstration of Leishmania tropica (LT) bodies in the skin slit smears/skin biopsies, 60 patients with cutaneous leishmaniasis were included in the study. The patients were randomly allocated to three groups of 20 patients each: group 1 treated with intramuscular injection of meglumine antimoniate (20 mg Sb/kg/day, maximum 850 mg) for 21 days; group 2 treated with intralesional injection of meglumine antimoniate (0.5 mL, 42.5 mg of Sb) into each lesion along with intramuscular injection of meglumine antimoniate (20 mg Sb/kg/day, maximum 850 mg) for 21 days; and group 3 as a control group. The patients were followed-up for therapeutic safety and efficacy at 10, 20 and 90 days. The rate of complete cure was 55% in group 1, 75% in group 2 and 10% of spontaneous cure cases in group 3. The conclusions derived from this study are limited by the relatively small number of patients. The combination of intramuscular meglumine antimoniate along with intralesional administration of the agent is superior therapy to intralesional administration of meglumine antimoniate alone.

Keratosis follicularis spinulosa decalvans associated with acne keloidalis nuchae and tufted hair folliculitis.

Keratosis follicularis spinulosa decalvans is a rare, X-linked disorder characterized by scarring alopecia of the scalp and eyebrows in the setting of widespread keratosis pilaris. Less frequent associations are ocular abnormalities and palmoplantar keratoderma. Acne keloidalis nuchae has previously been described in one patient with keratosis follicularis spinulosa decalvans. We report another case of keratosis follicularis spinulosa decalvans with acne keloidalis nuchae and tufted hair folliculitis, thus further establishing this association.

Dermatologic, periodontal, and skeletal manifestations of Haim-Munk syndrome in two siblings.

Haim-Munk syndrome is an extremely rare autosomal recessive disorder of keratinization characterized clinically by palmoplantar hyperkeratosis, severe early onset periodontitis, onychogryphosis, pes planus, arachnodactyly, and acro-osteolysis. Recently, germline mutations in the lysosomal protease cathepsin C gene have been identified as the underlying genetic defect in Haim-Munk syndrome and in the clinically related disorders, Papillon-Lefèvre syndrome and prepubertal periodontitis.

Feasibility and diagnostic agreement in teledermatopathology using a virtual slide system.

We investigated the feasibility and diagnostic agreement of a virtual slide system (VSS) in teledermatopathology. Forty-six biopsy specimens from inflammatory skin diseases were selected and scanned with a VSS at the Research Unit of Teledermatology, Medical University of Graz, Graz, Austria. Images were stored on a virtual slide server on which a specific Web application suited for telepathology (http://telederm.org/research/dermatopath/) runs. Twelve teleconsultants from 6 different countries reviewed the 46 cases, working directly on the Web application. Telediagnoses agreed with gold standard and conventional diagnosis with an average of 73% and 74%, respectively. Complete concordance among all teleconsultants with gold standard and conventional diagnosis was found in 20% of the cases. In 10 cases in which complete clinical data were missing, the average agreement of telediagnosis with gold standard diagnosis and conventional diagnosis decreased to 65% and 66%, respectively. Only 3 of 4 cases of inflammatory skin diseases were correctly diagnosed remotely with VSS. The system that we have used, despite its usability, is not completely feasible for teledermatopathology of inflammatory skin disease. Moreover, the performance seems to have been influenced by the availability of complete clinical data and by the intrinsic difficulty of the pathology of inflammatory skin diseases.

Piebaldism: a case report and a concise review of the literature.

Piebaldism is a rare autosomal dominant disorder characterized by congenital poliosis and leukoderma. We present a case of a 10-year-old girl with a typical clinical presentation, followed by a concise review of the literature discussing the etiology, clinical features, diagnosis, and management of the condition.

Tuberculosis verrucosa cutis presenting as an annular hyperkeratotic plaque.

GOAL: To understand cutaneous tuberculosis to better manage patients with the condition OBJECTIVES: Upon completion of this activity, dermatologists and general practitioners should be able to: 1. Recognize the morphologic features of cutaneous tuberculosis. 2. Describe the histopathologic characteristics of cutaneous tuberculosis. 3. Explain the treatment options for cutaneous tuberculosis.

Inflammatory linear verrucous epidermal nevus: a case report and short review of the literature.

GOAL: To understand inflammatory linear verrucous epidermal nevus (ILVEN) to better manage patients with the condition. OBJECTIVES: Upon completion of this activity, dermatologists and general practitioners should be able to: 1. Describe the presenting characteristics of ILVEN. 2. Explain the differential diagnosis of ILVEN. 3: Discuss the treatment options for ILVEN.

Granuloma inguinale: a case report.

Granuloma inguinale is common in certain regions of the world, however, it is rarely reported in the United States. It is the result of infection by Calymmatobacterium granulomati, although current literature proposes to re-classify this organism as Klebsiella granulomati. Here we report a case of granuloma inguinale, review the literature, and discuss historical context, treatment options, and differential diagnosis.

Papillon-Lefèvre syndrome: case report and review of the literature.

A 15-year-old boy presented with symmetric, well-demarcated, yellowish, keratotic plaques over the skin of his palms and soles extending onto the dorsal surfaces. Well-circumscribed, psoriasiform, erythematous, scaly plaques were also present on the elbows and knees bilaterally along with dystrophy an transverse grooving of the nails. He also had swollen and friable gums since the age of 3 with subsequent loss of most of his permanent dentition. These findings are consistent with Papillon-Lefèvre syndrome. The clinical presentation, differential diagnosis, complications and management of this syndrome are discussed.